Visit with the myasthenia specialist (my report)
 

First, I really got a kick out of seeing the plaque on his door. Right under his name it said "myasthenia gravis." Yay, a specialist!

Here's what he told me:

1) My SFEMG two years ago (by which I was diagnosed) was very strongly positive.

2) Hypokalemic Periodic Paralysis would not cause a positive SFEMG.

3) It's "common" for Mestinon not to help MG patients.

4) My spells of intense weakness are unusual for MG, but not weird enough for him to question my diagnosis because of them, especially since we've ruled out so many other things.

5) If Mestinon doesn't work, other acetylcholinesterase inhibitors won't, either, because they're very similar. (That said, I'm going to cautiously experiment with huperzine anyway--but remember, don't take huperzine if you're already taking Mestinon! They both inhibit the same enzyme!)

6) I should give the new higher dose of Imuran (I upped it to 250mg in October) another three months to kick in, and then consider either
Cyclosporin instead, or low-dose (10 or 20mg) Prednisone besides.

7) It's my hip muscles that are weakest. I could push back on his hand quite well in every test except the one where he had me sit on a chair and lift my knee up while he pushed down on it.

8) I don't present like someone who has a CMS, especially since I'm 46. Also, CMS's often give people a certain facial structure that I don't have.

9) He really thinks I have MG. It's true that a SFEMG isn't specific to MG, but the other diseases that yield positive results are nerve diseases that would have symptoms I don't have (I didn't ask what the diseases or symptoms are. I'm guessing it's stuff like numbness, pain, and tingling--just my guess).

That's all I can think of for now. My plan, subject to change, is to give the Imuran until June to kick in. The reason I'm waiting five months instead of three is that I got a huge boost in May last year. I want to see if it happens again, which might have something to do with getting more sun (yes, I'm taking vitamin D supplements). If not, I will consider the low-dose Prednisone.

Here's my thinking on the Prednisone (grateful acknowledgement to 4-Eyes for her part in this). There are two things about it that scare me: the immediate side effects (like weight gain, anxiety, and insomnia) and the long-term side effects (like diabetes, osteoporosis, and cataracts). The neuro suggested I try a low dose, and that might be enough, in conjunction with the Imuran. If I try it for a week and can't tolerate the immediate side-effects, I can quit. If I can tolerate them, I can hope to take it for a while (say, nine months) and then wean off it. It's something to consider.

Thanks to all who are following my continuing saga!

Abby

Very informative!!! I am on 20mg of Prednisone with no side effects but when I was on 80mg and 60mg it was horrible. I have borderline diabetes and take meds for it but not insulin, The 20mg has not changed that. My A1C test run 6.5 and varies very little.

I have gone downhill a little since middle of last week. My legs are always tired and I get short of breath pretty easy. Got my regular neuro visit in about an hour. Will see what he says.

Abby thanks for your great post!
Mike

glad it went well!!! and glad you saw someone who seems so knowledagble!!!

That sounds like great progress and info for your continued journey with slightly altered route......AWESOME :)

Anacrusis

Had my regular neuro visit today. Thank the good Lord for my wife 2 doctors missed my low level of Vit D. After she pointed that out he said to take 2000mg of D3 and reduced my Prednisone 2 1/2mg for 6 weeks. He said for me to call him if my legs or breathing got worse and we would IVIG again. I'm doing ok but could tell I had gone down hill a bit.
Mike

I'm glad you had a good appt. It sounds like it was very thorough. Except for one tiny little thing.

If looking at someone's facial structure was enough to diagnose them with a CMS, why spend all of that research money and time to develop all of those blood tests?! Come on!!! That's one of the biggest prejudices I've heard. That's like saying someone doesn't have MG if they don't have exaggerated ptosis. There is a CMS with facial malformations but that involves ONE mutation.

The doctor you saw was an MG expert, not a CMS expert. Yeah, there's a difference. I'm not saying you have CMS but there are so many variables within that group of genetic diseases that you can't say that age is the only factor. Or that facial structure is! :cool:

Have you tried physical therapy? Maybe they would be able to work with you to improve your hip area. It's possible. Or maybe your bed doesn't fit you well enough (seriously). I love how physical therapists can really help people with improving even the slightest problem.

Please also consider the cancer risk with combining two immuno's. It's a very real risk and the kind of cancers you can get, not to mention the "cure" for them, are much worse than relatively ok MG.

I just want you to have all of the facts during your decision making process. I hope you come up with a plan that works for you.

Annie

My neuro did say he wanted me to walk 3 times a weel plus my table tennis 3 times a week. I think he is testing my legs. My wife says the low vit D could cause my tiredness.
We didn't discuss my facial feature or CMS. You may have confused me with someone else. He did his usual tests, checked arm strength, leg strength and eye movement. He wants to see how I do this next couple of weeks. He thinks I may need anouther round of IVIG. I haven't had IVIG since Sep.
Mike

Annie, just to clarify: he didn't say that a certain facial structure, or lack thereof, is diagnostic. He mentioned it as one factor to consider among many in the decision about whether CMS is a real enough possibility to be worth a muscle biopsy at Mayo.

He didn't suggest I take cyclosporin and Imuran together. He suggested that I stop taking Imuran and start taking cyclosporin--or that I take Imuran and Prednisone together. When you talk about the cancer risk of combining to immunosuppressant drugs, did you mean Imuran and Prednisone? Or just Imuran and cyclosporin?

Physical therapy...I dunno, that's a really hard sell. For me to go to the appointment (half-hour drive into the city) would utterly exhaust me. It's hard for me to believe any benefit I could get could outweigh that.

Thanks for your help.

Abby

I meant steroids with Imuran or another immuno like Cellcept. It can be hard for the immune system to bounce back after a dynamic duo like that. I've seen it one too many times with people I've known who have lupus.

The muscle biopsy they do for MG is different than the one they do for CMS. I've had the basic one at Mayo - ouch! And you should know ahead of time that the CMS expert there "claimed" that they don't often see the NMJ in adults but only in children. That's a load of BS, however, since there are loads of articles on seeing it in adults! ;) The biopsy for CMS is far more dangerous because they normally take it from the intercostal muscles. They can take it from the arm too but both are considered "surgery" (under general anesthesia) while the MG one is more of a "procedure" (under local anesthesia).

At Mayo, they don't do the CMS biopsy much anymore. Neither do the CMS experts in Europe. They can tell from genetics, clinical history and blood tests if someone has a CMS versus MG. So that information your doc had is outdated. Again, I'm not trying to show him up or anything! It's just that you deserve to have the most accurate info in order to weigh everything. I'm not a doctor but I do pay attention to details more than some of them. :cool:

I wish I could put the big PDF I have from Hantai, an expert in Europe. It's the most informative one, besides the WUSTL site. I can't find it on the web either - I've tried.

I totally understand the PT thing. But what if you had a consult and then one follow up? You don't always have to have a bunch of appts. Or if you qualify for home health care, that might be a way to get around having to drive so far.

You have a great couple of neurologists and they're taking good care of you!

:hug:
Annie

Sounds like everyone had very productive visits with their neuro this week. I am so glad.

But now you have me curious. What are the facial features associated with CMS?

kathie